Classification of chronic liver disease

Practical needs of the clinic dictate the necessity of a detailed diagnosis of chronic liver diseases, which would be reflected all the main parties complex pathological process. In accordance with this requirement, and must be built classification of chronic liver disease. However, the systematization of these changes met with many difficulties. One of these is the lack of a clear distinction between different forms of diseases. So, expressed the value of immunological deviations reaction connective tissue liver formations in the progression of dystrophy of the liver, the commonality of many reasons make the division of chronic hepatitis and gepatocitami largely notional. The abundance of atypical forms of diseases, the diversity of terminology, the arbitrariness in the interpretation of the terms aggravate the situation. In addition, currently, cannot be satisfied with a separate classification of chronic hepatitis or cirrhosis. As these diseases are considered as a single stage of a pathological process, the obvious need to address chronic hepatitis, gepatocitami and liver cirrhosis in a single classification scheme.
The obvious and the need to build a classification on the basis of separate evaluation of the manifestations of pathological process, its etiological, morphology, clinical and functional aspects. The justification is that the same reason can cause various morphological changes in the liver; at the same time, the same morphological changes may be due to various reasons; often morphological changes are not adequately reflected in change of biochemical tests, and other clinical manifestations.
Depending on the prevalence of pathological changes in the liver parenchyma or connective tissue formations were considered epithelial and mesenchymal hepatitis (A. L. Myasnikov, 1956); hepatitis "mainly hepatocellular" and "predominantly mesenchymal" (with E. M. Tareev, 1965); destructive and infiltrative forms of hepatitis (A. F. of Bluger, 1968).
Within mainly interstitial liver lesions differ focal and diffuse hepatitis.
With a focal hepatitis affected part of the lobe of the liver (liver abscess) or multiple foci of changes can be scattered all over the body, as is the case when granulomatosa (tuberculosis, sarcoidosis, brucellosis, syphilis, parasitosis, some toxic-allergic damage).
Diffuse alopecia damage can be complicated diffuse involvement in the pathological process of liver cells, which leads to a significant disruption of the liver and makes a distinction between focal and diffuse hepatitis.
To predominantly interstitial hepatitis on the basis of leading damage to the connective tissue liver formations shall be assigned and hepatitis, developing as a result of disease of the bile ducts. But the fact that during its progression suffers parenchyma, disturbed architectonics of the liver and develops biliary cirrhosis leads to highlight holangiogepatit in a separate group.
Interstitial hepatitis, as diffuse (leishmaniasis, syphilis, subacute bacterial endocarditis), and focal represent an important section of Hepatology, however, the practical value them less than diffuse, progressing to cirrhosis of the liver diseases and above all true diffuse chronic hepatitis (with E. M. Tareev, 1965), where the inflammatory reaction of the connective tissue of the liver develops simultaneously with dystrophic changes in the parenchymal - "early conjugation and interstitial lung processes" (with E. N. Ter-Grigorova, 1950).
Proven relationship of this form of chronic hepatitis primarily with the epidemic hepatitis. The analysis of a large number of clinical, biochemical, morphological research suggests that the obvious progression is peculiar mainly chronic course of the epidemic hepatitis (with E. M. Tareev, 1958; X. X. Mansurov, 1962; Y. N. Darkseid, 1967, 1968; Popper, 1966; E. M. Tareev al., 1970).
Other etiological factors, such as infectious mononucleosis, toxico-allergic lesions (medicinal and developing in the "big collagenoses"), have much less of a role in the origin of this disease.
Cases of chronic hepatitis, developing gradually, without the acute onset, according to the nature of the biochemical and histological changes, obviously, are also mostly a consequence of the epidemic hepatitis, clinically latent flowing in the acute stage. It makes excessive offer Wepler (1961) division into primary and voicepresence forms that differ only in the background.
Based on clinical and morphological data, most clinicians identifies two forms of the disease: 1) persistent, benign, inactive and 2) aggressive, recurrent, chronic active hepatitis (with E. M. Tareev, 1965, 1970; Popper, 1968; A. S. Loginov, 1970, and others).
No doubt that the pace and rhythm progression of chronic hepatitis extremely diverse; in many cases, for 10-20 years does not occur significant restructuring of the architectonics of the liver and is not observed pronounced signs of liver dysfunction. However, the purity of this kind of liver damage is not given once for all, the quality of the disease. We repeatedly had to watch as in patients after long-term hepatitis without any signs of disease activity (and on this basis, identified as benign chronic persistent hepatitis) under the influence of various circumstances occurred active and rapid progression of the pathological process in the liver, very quickly led to the development of cirrhosis.
It is therefore rational to abandon the terms denoting not very consistent quality of the pathological process like this forever and several fatal sounding (":self-limiting", "improgressively" - A. F. of Bluger, 1968), and to consider active and inactive phase of the pathological process in the liver and stable or progressive course of the disease.
We fully share the well-reasoned opinion 3. , Prasinoi and I. E. Tareeva (1963) that, despite some features of the current so-called lupoid hepatitis, ploskokletochnom hepatitis and liver cirrhosis of young women, there is no reason to put them into a separate nosological group.
Most researchers, including first reported these forms of hepatitis, Moskau et al. (1956, 1968), consider them as a variant of chronic infectious hepatitis. The emergence LE-cells, hypergammaglobulinemia, plasmocytoma infiltration in the liver and bone marrow, the presence of serum antibodies to liver tissue indicate a high degree of immunological changes, occurring usually with chronic hepatitis and liver cirrhosis.
As for the so-called chronic cholestatic hepatitis, due to the peculiarities of morphological changes and clinical course, and in some cases cause the moment (along with the epidemic hepatitis - reaction to medicinal substances) this form can be considered as a stand-alone variant true diffuse hepatitis.
Thus, progressive diffuse hepatitis presents the following morphological forms; true diffuse chronic hepatitis, holangiogepatit and cholestatic hepatitis.
Currently, according to the classification developed by V pan-American Congress of gastroenterology in Havana (1956), the following sub-clinical-morphological types of cirrhosis: postnecrotic, portal, biliary (obturation with extrahepatic bile duct and without it) and mixed.
It is possible to distinguish three stages of development of cirrhosis: primary, stage formed of cirrhosis, end (dystrophic) stage. Morphological characteristics of the stages listed above, with the description of pathomorphological changes each type of liver cirrhosis.
Clinical-morphological comparison shows that during the first two stages of the development of cirrhosis of liver dysfunction due to degenerative and necrobiotic changes in the parenchyma, resulting in progression of the pathological process. In periods of relative inactivity pathological process surviving and regenerierbetrieb hepatocytes in some extent compensate for poor liver function. In the end these violations irreversible.
For diffuse liver diseases can be different. There are progressing, stabilization and devolution (reverse development) pathological changes. The progression of the disease occurs in different ways by tempo and character. There are 5 options for the development of chronic hepatitis. Last may progress continuously and quickly, sometimes slowly evolving to cirrhosis over several years. Often chronic hepatitis develops in retidiviruuschem option, as the number of exacerbations, alternating with periods of stabilization of the pathological process.
Vary the pace of evolution and dystrophy of the liver, particularly fat. In most cases, the progression is slow. Rare is the rapid development of cirrhosis based fatty degeneration. Popper, Szanto, Parthasarathy (1955) and Steiner (1960) attach so much importance to the pace of evolution of the disease that quickly transforms "fatty liver" in cirrhosis describe as an independent form of so - called "blooming cirrhosis" ("florid cirrhosis").
Since most often the progression of chronic hepatitis and liver cirrhosis occurs retidiviruuschem type, it is necessary to distinguish between active and inactive phase of the pathological process.
Morphological evidence of active disease process in the liver are: the extension collagenase portal and periportal fields, penetration of cellular infiltrates and collagen fibers of the periportal zones inside cloves, destruction of the bounding plate; severe degeneration of hepatocytes, focal necrosis them mainly on the periphery of cloves; active regeneration appearing in anisocytosis of hepatocytes and their nuclei, the emergence of multi-core liver cells; activation coppersky cells (X. X. Mansurov, S. N. Koutchak, 1964; Steiner, 1964; De Groote, Schmid, 1968).
Clinical signs of activity of the process are unwell, pain in the right hypochondrium and epigastric pain, a manifestation of new star telangiectasia. Often when morphologically very active hepatitis does not occur expressed clinical manifestations.
The most convincing biochemical a sign of progression of chronic liver disease is the determination of activity of enzymes in the serum. These tests, in the words Wroblewski (1958), are set to "Entomologicheskoe liver biopsy and help to detect chronic liver disease and the establishment of the activity of the pathological process. The increasing transaminaz, alkaline phosphatase and fall of cholinesterase activity in the serum often ahead of all other clinical manifestations. However, in 12-18% of cases the definition of these enzymes in the serum does not detect an existing liver chronic active hepatitis.
More clearly, the activity of the pathological process in the liver reflect changes arginase activity (E. M. Tareev al., 1970), ornithine-carbamoyltransferase, fructose-1-hospitalilty and increase of activity of 5-th fraction lactate dehydrogenase (G I. Barsukova and S. D. Podymova; 3. A. Bondar, 1970).
In most cases when activity is increasing the number ' -globulinov and decreases the amount of albumin in the blood serum. Decline sharply alpha-lipoprotein, sometimes they are not determined; increases the content of alpha-glycoproteins, especially A2-faction; reduced glycoproteins in albumen fraction (I. D. Mansurov and others, 1963).


Change often results of functional tests; in the majority of patients increased levels of bilirubin in the blood, but in those cases, where the concentration of bilirubin in the blood is normal, along with the free bilirubin, you monoglucuronide-bilirubin (3. D. Schwarzmann, 1963).
On the activity of chronic hepatitis shows a marked change bromsulfaleinovy samples. At present more and more importance in terms of identifying active chronic hepatitis given immunologic investigations: detection of antinuclear antibodies (Renger, 1969), antibodies to mitochondria and other components of liver cells.
At stabilization of the pathological process dynamics of clinical manifestations depend on the stage, which was halted. In the early stages disappear subjective symptoms, the results of functional tests are aligned to normal. In the stage formed cirrhosis improvement in the subjective and objective indicators to be less complete, end-stage cirrhosis of the liver remain broken, regardless of the process activity.
Therefore, more reliable biochemical criteria of stabilization is the normalization of activity of enzymes, sometimes normal content ' -globulins in the blood.
Histological signs of stabilization are the absence of evidence of active regeneration parenchyma, the clarity of boundaries between parenchyma and connective tissue bands, the absence or low level of the inflammatory response, the absence of necrosis.
Finding out the degree of dysfunction of liver cells by clinical and laboratory parameters in patients with cirrhosis of the liver is significant difficulties. In the presence of portal hypertension become insolvent stress tests, in particular trial of Quick - Pytel, sample diabetic load. Due to the presence of venous collaterals part substances - load - can penetrate into the General bloodstream, bypassing the liver. The same circumstances explain the emergence of urobilinemia while remaining functions of the liver cells. At some stages jaundice may not depend on the destruction of liver cells, and to be the result of hemolysis or beletaza. The most reliable signs of violation of the functions of the liver cells are changes in the ratio of protein fractions of blood plasma, decreased prothrombin index, the presence in the blood serum of mono - or diglucuronide (3. D. Schwarzmann, 1962, 1963), change holedoholitijazom factor in bile (A. N. Ardamatskaya, 1963), sublimate reaction (A. F. of Bluger, 1964), bromsulfaleinovy samples, rates of gepatopatia using labeled Bengal rose (A. S. Loginov with al., 1969; 3. A. Bondar, 1970; Sheppard and other, 1947).
B. N. Alferov (1960), A. F. of Bluger (1964) and others point to the increased content of iron in the blood serum as a very sensitive functional liver tests.
Along with the assessment of the performance functional tests crucial importance is the study of the General condition of the patient.
From a practical point of view, the assessment of the functional activity of the liver is expedient for carrying out on the following parameters:
I. Functional compensation: state of health is satisfactory, the results of functional tests are normal, normal levels of protein in the plasma, the lack of dysproteinemia; serum only free bilirubin; the content of urobilin in urine slightly increased.
II. Functional failure of the liver cells (hepatocellular insufficiency):
1) easy degree: the lower efficiency, weakness, indicators liver samples almost do not differ from the norm; prothrombin index is not reduced, the protein content of plasma normal or increased; increased levels of gamma-globulins, reduced the number of alpha-lipoprotein, increased fraction ' -glycoproteins; serum appears monoglucuronide bilirubin; urobilinemia;
2) the average degree: weakness, anorexia, dyspepsia, sometimes jaundice; main indicators of liver samples violated; prothrombin index reduced and not fully aligned after the introduction vikasola; the protein content of plasma normal or increased; the amount of albumin reduced; the level of gamma-globulin is increased; albumin-globulin ratio significantly reduced (0.7 and below); reduced or absent alpha-lipoprotein, increased content of alpha-glycoproteins; factor esterification cholesterol dropped; in serum mono - and diglucuronide bilirubin; expressed urobilinemia;
3) severe: intoxication syndrome with change of consciousness (precoma and coma), oliguria; a significant violation of the main indicators of the functional liver samples, sometimes intensive jaundice; a significant decrease in the prothrombin index, resistant to the introduction of vitamin K, a tendency to haemorrhage; changes protein formula plasma are similar to those with moderate malnutrition; often hypoproteinemia; in the most severe cases, reduces the content of gamma-globulins, increased residual nitrogen, indican, urea in the blood.
Assessment of the status of the portal blood flow is given in accordance with gradation offered N. P. Napalkov (see "Portal hypertension").
In addition to the characteristics of cirrhosis on the basic characteristics, it is necessary to pay attention to any signs of hypersplenism. The appearance of the latter largely determines the tactics of conservative and surgical treatment of a patient with cirrhosis of the liver.
All dismantled provisions relating to the classification of chronic diffuse diseases of the liver, can be combined in the classification scheme (table. 8).

TABLE 8. Classification scheme chronic diffuse liver diseases
Classification features Chronic hepatitis Liver dystrophy Cirrhosis
Morphological True diffuse

Holangiogepatit cholestatic
Fat

Glycogens, gemosideros, etc.
Portal
Postnecrotic
Biliary:
a) to extrahepatic obstruction (secondary);
b) without her (primary)
Mixed
Etiological Botkin's disease, cholangitis, obturation of the extrahepatic bile ducts, chlorpromazine, C17-substituted steroids Protein and vitamin deficiency (Exo - and endogenous), endocrine and metabolic disorders (diabetes, obesity, etc.), disorders of enzymatic processes
Unbalanced diet, alcoholism, kwashiorkor
Intoxication (including professional)
Reliable: Botkin's disease, malnutrition (Exo - and endogenous, including alcoholism), obturation of the extrahepatic bile ducts, blood circulation disorders, congenital syphilis, intoxication (carbon tetrachloride, trinitrotoluene, arsenic compounds, hairy heliotrope), toxico-allergic.
Less reliable: granulematosny, helminthes (opisthorchiasis, schistosomiasis), abnormalities in the metabolism of copper and iron

Clinical characteristics:

1. Over and phase
A. Progressive:
a) continuous, rapid; b) a continuous, slow;
in) relapsing, during the last phase of active and inactive
B. Stable
Century Regressing
A. Progressive:
a) slowly;
b) just

B. Stable
Century Regressing
A. Progressive: a) the phase of active;

b) phase inactive

B. Stable
2. The stage of the disease (prevalence and depth of changes)     Initial
Formed cirrhosis
End (dystrophic)
3. The functions of the liver cells A. Compensated
B. Failure
hepatocellular:
1) mild;
2) the average degree;
3) heavy
The same The same
4. State portal blood flow - - A. the Nature of the block:
1) intrahepatic;
2) mixed
B. Type the portal hypertension:
a) total;
b) total with prevalence of intestinal-mesenteric;
C) total dominated rastrelyannogo
B. Payment of a block of collaterals:
1) compensated;
2) subcompensated;
3) decompensated
5. Hypersplenism There Is No   There Is No

Since the distinction between the portal and postnecrotic cirrhosis often very difficult, and in the final stage of the disease is simply impossible, should be considered optional differentiation of these two morphological types of the disease, but delimitation them from biliary cirrhosis is absolutely necessary.
Of course, that a detailed diagnosis of the disease in accordance with this classification scheme can be put only in the conditions of specialized Department, which features a special instrumental examination methods. In other conditions, may establish the existence of chronic liver disease, tentative definition stage (chronic hepatitis, cirrhosis), judgment on the activity of the pathological process, a rough estimation of the functional state of the liver and portal hypertension.